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ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Volume 39 Issue 2
Feb.  2023
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Article Navigation >  Journal of Clinical Hepatology  > 2023  >  39(2): 365-369

Clinical features of myeloproliferative neoplasms with portal hypertension as the main manifestation

DOI: 10.3969/j.issn.1001-5256.2023.02.017

  • Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China

Research funding:

Captial's Funds for Healthy Improvement and Research (2022-2-1104);

Digestive Medical Coordinated Development Center of Beijing Hospitals Authority (XXT04)

More Information
  • Corresponding author: WANG Yu, wangyuliver@ccmu.edu.cn (ORCID: 0000-0003-1800-2082)
  • Received Date: 2022-07-30
  • Accepted Date: 2022-09-13
  • Published Date: 2023-02-20
    Abstract
  •   Objective  To investigate the clinical features, liver histological features, and diagnostic and treatment methods for patients with myeloproliferative neoplasms (MPN) with portal hypertension as the main manifestation.  Methods  A retrospective analysis was performed for related data of the patients who attended the hospital due to portal hypertension and were finally diagnosed with MPN in Liver Research Center, Beijing Friendship Hospital, from January 2019 to February 2022, including clinical manifestation, liver pathological features, treatment, and follow-up results.  Results  Nine patients were included in this study, and all the patients had splenomegaly and esophageal and gastric varices, while portal vein thrombosis was observed in eight patients. All patients had normal or slightly abnormal liver function and routine blood test results. Six patients underwent liver biopsy, without the formation of fibrous septum and pseudolobule, and hepatic extramedullary hematopoiesis was observed in two patients. All nine patients underwent bone marrow biopsy and genetic testing, among whom six had essential thrombocythemia and three had primary myelofibrosis, and genetic testing revealed JAK-2V617F gene mutation in seven patients and CALR gene mutation in two patients.  Conclusion  MPN is one of the rare causes of portal hypertension and has the clinical manifestations of esophageal and gastric varices, splenomegaly, and even megalosplenia, without the manifestations of hypersplenism such as leukopenia and thrombocytopenia. Detection of the JAK-2V617F and CALR genes can improve the diagnostic rate of MPN.

     

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    • References(15)
  • [1]
    THATIPELLI MR, MCBANE RD, HODGE DO, et al. Survival and recurrence in patients with splanchnic vein thromboses[J]. Clin Gastroenterol Hepatol, 2010, 8(2): 200-205. DOI: 10.1016/j.cgh.2009.09.019.
    [2]
    AGENO W, RIVA N, SCHULMAN S, et al. Antithrombotic treatment of splanchnic vein thrombosis: results of an international registry[J]. Semin Thromb Hemost, 2014, 40(1): 99-105. DOI: 10.1055/s-0033-1363473.
    [3]
    BARBUI T, THIELE J, GISSLINGER H, et al. The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion[J]. Blood Cancer J, 2018, 8(2): 15. DOI: 10.1038/s41408-018-0054-y.
    [4]
    LAVU S, SZUBER N, MUDIREDDY M, et al. Splanchnic vein thrombosis in patients with myeloproliferative neoplasms: The Mayo clinic experience with 84 consecutive cases[J]. Am J Hematol, 2018, 93(3): E61-E64. DOI: 10.1002/ajh.24993.
    [5]
    SANT'ANTONIO E, GUGLIELMELLI P, PIERI L, et al. Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international, retrospective study on 518 cases[J]. Am J Hematol, 2020, 95(2): 156-166. DOI: 10.1002/ajh.25677.
    [6]
    FINAZZI G, DE STEFANO V, BARBUI T. Splanchnic vein thrombosis in myeloproliferative neoplasms: treatment algorithm 2018[J]. Blood Cancer J, 2018, 8(7): 64. DOI: 10.1038/s41408-018-0100-9.
    [7]
    ABU-HILAL M, TAWAKER J. Portal hypertension secondary to myelofibrosis with myeloid metaplasia: a study of 13 cases[J]. World J Gastroenterol, 2009, 15(25): 3128-3133. DOI: 10.3748/wjg.15.3128.
    [8]
    WANG J, LIU XF, TIE J. Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension[J]. J Clin Hapatol, 2021, 37(11): 2617-2621. DOI: 10.3969/j.issn.1001-5256.2021.11.025.

    王静, 刘晓锋, 帖军. 原发性骨髓纤维化合并肝血管性疾病和/或门静脉高压症的临床特征和预后[J]. 临床肝胆病杂志, 2021, 37(11): 2617-2621. DOI: 10.3969/j.issn.1001-5256.2021.11.025.
    [9]
    de FRANCHIS R, BOSCH J, GARCIA-TSAO G, et al. Baveno Ⅶ-Renewing consensus in portal hypertension[J]. J Hepatol, 2022, 76(4): 959-974. DOI: 10.1016/j.jhep.2021.12.022.
    [10]
    KLAMPFL T, GISSLINGER H, HARUTYUNYAN AS, et al. Somatic mutations of calreticulin in myeloproliferative neoplasms[J]. N Engl J Med, 2013, 369(25): 2379-2390. DOI: 10.1056/NEJMoa1311347.
    [11]
    POISSON J, PLESSIER A, KILADJIAN JJ, et al. Selective testing for calreticulin gene mutations in patients with splanchnic vein thrombosis: A prospective cohort study[J]. J Hepatol, 2017, 67(3): 501-507. DOI: 10.1016/j.jhep.2017.04.021.
    [12]
    TAN HK, LEOW WQ, CHANG PE. Ruxolitinib for the treatment of portal hypertension in a patient with primary myelofibrosis[J]. Gastroenterology, 2019, 157(5): e26-e27. DOI: 10.1053/j.gastro.2016.08.059.
    [13]
    NAYMAGON L, TREMBLAY D, ZUBIZARRETA N, et al. Portal vein thrombosis patients harboring JAK2V617F have poor long-term outcomes despite anticoagulation[J]. J Thromb Thrombolysis, 2020, 50(3): 652-660. DOI: 10.1007/s11239-020-02052-4.
    [14]
    ZHANG JB, CHEN J, ZHOU J, et al. Systematic review and meta-analysis of trans-jugular intrahepatic portosystemic shunt for cirrhotic patients with portal vein thrombosis[J]. World J Clin Cases, 2021, 9(19): 5179-5190. DOI: 10.12998/wjcc.v9.i19.5179.
    [15]
    REILLY CR, BABUSHOK DV, MARTIN K, et al. Multicenter analysis of the use of transjugular intrahepatic portosystemic shunt for management of MPN-associated portal hypertension[J]. Am J Hematol, 2017, 92(9): 909-914. DOI: 10.1002/ajh.24798.
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